High risk and low prevalence diseases: Lemierre's syndrome.

INTRODUCTION: Lemierre's syndrome is a serious condition that carries with it a high rate of morbidity and even mortality. OBJECTIVE: This review highlights the pearls and pitfalls of Lemierre's syndrome, including presentation, diagnosis, and management in the emergency department (ED) based on current evidence. DISCUSSION: Lemierre's syndrome is a condition marked by septic thrombophlebitis of the internal jugular vein (IJV), with the classic triad of pharyngotonsillitis, IJV thrombosis, and septic emboli resulting in metastatic abscess. It typically begins as pharyngitis, often caused by Fusobacterium necrophorum. Patients most commonly present with fever, recently diagnosed pharyngitis, and neck pain or swelling. Septic emboli may affect multiple organ systems, most commonly the pulmonary system. The disease should be considered in patients with prolonged symptoms of pharyngitis, pharyngitis that improves but then worsens, critically ill patients with pharyngitis, patients with pharyngitis and infection at a secondary site, and neck signs/symptoms. Diagnosis includes throat and blood cultures, as well as imaging to include computed tomography of the neck and chest with intravenous contrast. Additional imaging of other areas should be performed as clinically indicated. Initial management includes hemodynamic stabilization with intravenous fluids and vasopressors as needed, as well as broad-spectrum antibiotics. Anticoagulation for the primary thrombus and possible septic emboli is controversial and should be considered in a multidisciplinary approach with admission. CONCLUSIONS: An understanding of Lemierre's syndrome can assist emergency clinicians in diagnosing and managing this potentially deadly disease.

Cardiac and Cerebral Arterial Complications of Lemierre Syndrome: Results from a Systematic Review and Individual Patient Data Meta-analysis.

BACKGROUND: Lemierre syndrome is a potentially life-threatening disease, which affects otherwise healthy young adults and adolescents. It is characterized by acute neck vein thrombosis and septic embolism, usually complicating a bacterial infection. Data on the syndrome are sparse, particularly concerning arterial complications. METHODS: We evaluated the frequency and patterns of cerebral arterial and cardiac involvement ("arterial complications") in an individual patient level cohort of 712 patients, representing all cases described over the past 20 years in the medical literature who fulfilled the criteria: (1) bacterial infection in the neck/head site and (2) objectively confirmed thrombotic complication or septic embolism. The study outcomes were defined as all-cause in-hospital deaths and the occurrence of clinical sequelae at discharge or in the postdischarge period. RESULTS: A total of 55 (7.7%) patients had an arterial complication. The most frequent arterial complications were carotid involvement (52.7%), stroke (38.2%), and pericardial complications (20%). Patients with an arterial involvement were more likely to be treated with a greater number of antibiotics (23 vs. 10%) and to receive anticoagulation. In addition, patients with arterial complications had a greater risk of all-cause death (n = 20/600, 3.3% vs. n = 6/52, 12%; odds ratio [OR]: 3.8; 95% confidence interval [CI]: 1.5-9.9) and late clinical sequelae (n = 49/580, 9.0% vs. n = 15/46, 35%; OR: 5.2; 95% CI: 2.65-10.37). CONCLUSIONS: While Lemierre syndrome is known to be primarily characterized by venous thromboembolic events, our results suggest that local or distant arterial complications may occur in approximately one-tenth of patients and may be associated with a greater risk of long-term sequelae and death.

Lemierre's syndrome in adulthood, a case report and systematic review.

Introduction: Lemierre's syndrome is a septic thromboembolic complication of an oropharyngeal or neck infection, primarily caused by Fusobacterium species. Although it usually affects young healthy patients, some case reports describe this syndrome in older population.Methods: A case report and a systematic review of the literature were conducted to investigate the late onset of Lemierre's syndrome. Forty-one articles were selected for the qualitative analysis, 39 for the quantitative analysis.Results: The average age of the study population was 52 years old. Diabetes mellitus and upper gastro-intestinal malignancy, common comorbidities in the study population, might play a role in the development of late-onset Lemierre's syndrome. Empiric antibiotic treatment should cover Fusobacterium and Streptococcus species both, which may cooperate to induce purulent disease. Reported unfavourable outcome was more than expected.Conclusion: Lemierre's syndrome in adulthood may differ from the usual version. This disease may further pass unrecognized, if presented out of the expected age range. Nevertheless, early diagnosis and prompt treatment are a requisite to prevent morbidity and mortality, which may be higher in this older population.

Lemierre's syndrome in the pediatric population: Trends in disease presentation and management in literature.

OBJECTIVE: This study aims to examine literature on Lemierre's Syndrome (LS) in the pediatric population over time in order to describe patterns in disease progression, management, and prognosis. In addition, this study assesses specific rate of literature output and the location of research over the past 10 years. METHODS: A literature review was conducted through two databases, PubMed.gov and PMC. A search was conducted using the keywords "Lemierre syndrome" and "postanginal sepsis." Literature was primarily reviewed for demographic, radiographic, and clinical data. Articles were included in the study if they were published in English and within the last 10 years. All types of research studies were recorded, however primary data collection came from case reports and series. Publications were grouped into two time periods: 2009-2013 and 2014-2019, allowing for comparison of various characteristics between these two groups. RESULTS: A total of 124 research studies on LS met inclusion criteria and were reviewed. Of these, 98 case reports (79.0%) were examined. Disease Characteristics: Fusobacterium necrophorum was the most common precipitating pathogen isolated from cultures (66.2%). The main primary treatment modalities used were antimicrobials, surgery, anticoagulation, or a combination of these treatments. A total of 63.9% of the case reports indicated use of anticoagulation at some point during treatment. Publication Trends: The number of published studies has not significantly changed in the last decade, with a non-statistically significant decline of 5.6%, when comparing 2014-2019 to 2009-2013 (p = 0.21). Case reports/series were the most common study design (82.2% vs 69.5%) and level of evidence for published studies continued to be stable (level 4-5) through the years (86.9%). The number of publications within an international journal vs US based journal has also remained steady during both time periods (p = 0.698). CONCLUSION: LS is an uncommon condition but one that is important for physicians to be aware of in the pediatric population. Treatment regimens including antibiotics and anticoagulation have remained stable through the past 10 years, however the efficacy of anticoagulation in treating LS continues to be debated. Though LS is considered a severe illness with potentially life threatening complications, publications on this topic, in pediatrics specifically, have decreased within the past five years.

Head, Neck, and Abdominopelvic Septic Thrombophlebitis: Current Evidence and Challenges in Diagnosis and Treatment.

Septic thrombophlebitis (STP) is a complex, cross-disciplinary clinical condition that combines a localized infection with a neighboring venous thrombosis. STP can occur at several possible anatomic sites, such as dural sinuses, jugular vein (Lemierre syndrome), portal vein (pylephlebitis), and pelvic veins. Its high mortality in the preantibiotic era improved considerably with the introduction of modern antibiotics. However, little evidence exists to date to guide its clinical management. The incidence of STP or its risk factors may be increasing, and its mortality may still be considerable. These trends would have far-reaching implications, especially in the setting of increasing resistance to antimicrobial agents. No clinical assessment tools exist to support patient screening or guide treatment in STP. Few interventional studies exist on the efficacy and safety of anticoagulation. Recommendations on its indications, duration, and the agents of choice are mostly based on evidence derived from small observational studies. While all forms of STP pose similar challenges, future research may benefit from the distinction between bacteria-associated, virus-associated, and mycosis-associated thrombophlebitis. Addressing these gaps in evidence would enhance our ability to diagnose this condition and treat patients effectively.

Invasive infections with Fusobacterium necrophorum including Lemierre's syndrome: an 8-year Swedish nationwide retrospective study.

OBJECTIVES: We aimed to evaluate the nationwide incidence and a potential increase in invasive infections with Fusobacterium necrophorum. Secondly, we aimed to describe epidemiology, clinical characteristics and outcomes for the different presentations: Lemierre's syndrome (LS), invasive head and neck-infection without LS and invasive non-head and neck-infection. METHODS: A retrospective multicentric population-based study of all invasive infections with F. necrophorum diagnosed in Sweden from 2010 to 2017 with 6 months of follow-up was performed through reviews of medical records. Invasive infections were defined and identified by a positive blood culture or sequencing of 16S rDNA, targeted PCR or culture from normally sterile sites. Incidence calculations were performed, including comparisons between 2010-13 and 2014-17, age groups and clinical presentations. Patient and infection characteristics, treatment and clinical outcomes were analysed. RESULTS: Invasive infections with F. necrophorum were diagnosed in 300 cases in Sweden 2010-17. The incidence increased from 2.9 to 5.0 cases/million/year from 2010-13 to 2014-17 (p 0.001). A total of 104/300 (35%) patients developed LS, 102/300 (34%) invasive head and neck infection without LS and 94/300 (31%) invasive non-head and neck infection. The median age was 20, 25 and 64 years, respectively. Among patients with LS 72/96 (75%) had thrombocytopenia on admission, 86/104 (83%) had sepsis, 19/104 (18%) developed septic shock and 45/104 (43%) needed intensive care. 30-day mortality in LS was 2/104 (2%). CONCLUSION: We describe an increased incidence of invasive infections with F. necrophorum in Sweden and highlight its full spectrum of invasive clinical presentations. LS, in particular, causes considerable morbidity in young and previously healthy patients.

[A sepsis that you don't expect Lemierre's syndrome. Description of a clinical case.] / Una sepsi che non ti aspetti la sindrome di Lemierre. Descrizione di un caso clinico.

A healthy 16-year-old boy was hospitalized for fever, septic condition and thrombosis of the left internal jugular vein: The diagnosis of Lemierre syndrome (LS) with positive blood culture for Fusobacterium necrophorum was formalized. He was treated with antibiotics and anticoagulant therapy with enoxaparin with complete recovery. Four weeks after discharge, the jugular vein ecodoppler showed complete resolution of the thrombosis. LS is characterized by thrombosis of the internal jugular vein and anaerobic bacteremia often caused by Fusobacterium necrophorum. It is a rare disease in the post-antibiotic era, but with an increase in cases over the last twenty years. LS should be suspected in young, healthy patients with persistent pharyngitis followed by sepsis, pneumonia or atypical laterocervical pain. The diagnosis is confirmed by the identification of jugular venous thrombosis and is further confirmed by the growth of anaerobic bacteria on blood culture. Prolonged antibiotic and anticoagulant therapies are the essential elements of treatment. However, anticoagulant therapy for internal venous thrombosis associated with LS remains a controversy.

Thrombosis of the internal jugular vein in the ENT-department - Prevalence, causes and therapy: A retrospective analysis.

OBJECTIVE: Less than 5% of deep vein thrombosis is due to thrombosis of the internal jugular vein. Genetic, malignant or inflammatory underlying diseases as well as insertion of venous catheters can be responsible for this pathology. Due to its rare occurrence, it is difficult to find systematic research about thrombosis of the internal jugular vein. METHODS: We performed a systematic analysis of present patient data from our ENT department with the electronic patient record considering the period from 2012-2017. Search terms were "thrombosis" and "jugular internal vein". We identified 41 patients with the requested diagnosis and performed further analysis of the cases. Internal jugular vein thrombosis was diagnosed in all patients using Duplex sonography and/or CT/MR angiography. RESULTS: Paraneoplastic thrombosis was found in 22/41 patients (54%), in 15 of the 22 (68%), the tumor was located in the ENT region. Two out of seven (29%) of the patients with tumor entities outside the head and neck region had thrombosis of the internal jugular vein as the first symptom of the disease. Another 14/41 patients (34%) had underlying inflammatory diseases - mostly streptococci-associated - for example a cervical abscess. In two patients, insertion of a central-venous catheter was causal, in three patients we could not find any reason for the development of thrombosis. CONCLUSION: To diagnose the rare and often asymptomatic thrombosis of the internal jugular vein, ultrasound of the cervical region should always include vascular imaging. Thrombosis of the internal jugular vein results mostly paraneoplastic or due to inflammation/abscess. It can be the first symptom of a malignant primary disease and always requires detailed diagnostic clarification. LEVEL OF EVIDENCE: 4.

The long shadow of lemierre's syndrome.

Lemierre's syndrome is a rare and feared complication of pharyngitis, occurring most commonly in adolescents and young adults. It is typically defined by the constellation of septic internal jugular vein thrombophlebitis, pulmonary and other septic emboli, and sterilesite infection by Fusobacterium necrophorum. The rarity and severity of Lemierre's syndrome has made it an attractive subject for case reports but there is a paucity of evidence to inform areas of persistent uncertainty. In recent years, heightened attention and controversy has focused upon speculation that a purported rise in the incidence of Lemierre's syndrome is due to reduced antibiotic prescribing for respiratory tract infections, that F. necrophorum is an under-appreciated cause of acute tonsillopharyngitis and that testing and targeted treatment would prevent cases of Lemierre's syndrome.

Safety of reduced antibiotic prescribing for self limiting respiratory tract infections in primary care: cohort study using electronic health records.

OBJECTIVE:  To determine whether the incidence of pneumonia, peritonsillar abscess, mastoiditis, empyema, meningitis, intracranial abscess, and Lemierre's syndrome is higher in general practices that prescribe fewer antibiotics for self limiting respiratory tract infections (RTIs). DESIGN:  Cohort study. SETTING:  610 UK general practices from the UK Clinical Practice Research Datalink. PARTICIPANTS:  Registered patients with 45.5 million person years of follow-up from 2005 to 2014. EXPOSURES:  Standardised proportion of RTI consultations with antibiotics prescribed for each general practice, and rate of antibiotic prescriptions for RTIs per 1000 registered patients. MAIN OUTCOME MEASURES:  Incidence of pneumonia, peritonsillar abscess, mastoiditis, empyema, meningitis, intracranial abscess, and Lemierre's syndrome, adjusting for age group, sex, region, deprivation fifth, RTI consultation rate, and general practice. RESULTS:  From 2005 to 2014 the proportion of RTI consultations with antibiotics prescribed decreased from 53.9% to 50.5% in men and from 54.5% to 51.5% in women. From 2005 to 2014, new episodes of meningitis, mastoiditis, and peritonsillar abscess decreased annually by 5.3%, 4.6%, and 1.0%, respectively, whereas new episodes of pneumonia increased by 0.4%. Age and sex standardised incidences for pneumonia and peritonsillar abscess were higher for practices in the lowest fourth of antibiotic prescribing compared with the highest fourth. The adjusted relative risk increases for a 10% reduction in antibiotic prescribing were 12.8% (95% confidence interval 7.8% to 17.5%, P<0.001) for pneumonia and 9.9% (5.6% to 14.0%, P<0.001) for peritonsillar abscess. If a general practice with an average list size of 7000 patients reduces the proportion of RTI consultations with antibiotics prescribed by 10%, then it might observe 1.1 (95% confidence interval 0.6 to 1.5) more cases of pneumonia each year and 0.9 (0.5 to 1.3) more cases of peritonsillar abscess each decade. Mastoiditis, empyema, meningitis, intracranial abscess, and Lemierre's syndrome were similar in frequency at low prescribing and high prescribing practices. CONCLUSIONS:  General practices that adopt a policy to reduce antibiotic prescribing for RTIs might expect a slight increase in the incidence of treatable pneumonia and peritonsillar abscess. No increase is likely in mastoiditis, empyema, bacterial meningitis, intracranial abscess, or Lemierre's syndrome. Even a substantial reduction in antibiotic prescribing was predicted to be associated with only a small increase in numbers of cases observed overall, but caution might be required in subgroups at higher risk of pneumonia.

Septic thrombosis of the internal jugular vein: Lemierre's syndrome revisited.

OBJECTIVES/HYPOTHESIS: Study of the clinical evolution of a primary ear, nose, and throat infection complicated by septic thrombophlebitis of the internal jugular vein. STUDY DESIGN: Retrospective case-control study. PATIENTS AND METHODS: From 1998 to 2010, 23 patients at our institution were diagnosed with a septic thrombosis of the internal jugular vein. Diagnostics included microbiologic analysis and imaging such as computed tomography, magnetic resonance imaging, and ultrasound. Therapy included broad-spectrum antibiotics, surgery of the primary infectious lesion, and postoperative anticoagulation. The patients were retrospectively analyzed. RESULTS: The primary infection sites were found in the middle ear (11), oropharynx (8), sinus (3), and oral cavity (1). Fourteen patients needed intensive care unit treatment for a mean duration of 6 days. Seven patients were intubated, and two developed severe acute respiratory distress syndrome. An oropharynx primary infection site was most prone to a prolonged clinical evolution. Anticoagulation therapy was given in 90% of patients. All 23 patients survived the disseminated infection without consecutive systemic morbidity. CONCLUSION: In the pre-antibiotic time, septic internal jugular vein thrombophlebitis was a highly fatal condition with a mortality rate of 90%. Modern imaging techniques allow early and often incidental diagnosis of this clinically hidden complication. Anticoagulation, intensive antibiotic therapy assisted by surgery of the primary infection site, and intensive supportive care can reach remission rates of 100%.

Lemierre's syndrome: the importance of early detection.

Sore throat is a common complaint, generally thought to be viral in origin, for which there may be a reluctance to prescribe antibiotics. This, combined with the emergence of antibiotic resistance, may explain the recent rise in the number of reports of Lemierre's syndrome (LS). LS characterises a postanginal septicaemia that is associated with significant morbidity and mortality if not recognised and treated early. This article describes the management of a 17-year-old boy diagnosed with LS to illustrate its classical presentation, common pitfalls in diagnosis and optimal management.

Lemierre syndrome.

Lemierre's syndrome is an uncommon complication of pharyngitis in the United States and caused most commonly by the bacterium Fusobacterium necrophorum. The syndrome is characterized by a history of recent pharyngitis followed by ipsilateral internal jugular vein thrombosis and metastatic pulmonary abscesses and is a disease for which patients will seek medical care and advice. As most patients are admitted to the hospital under internal medicine, practitioners should be familiar with the usual signs and symptoms of Lemierre's syndrome along with its diagnosis and treatment. Controversy involves the choice and duration of antimicrobial therapy used for treatment and anticoagulation therapy for internal jugular vein thrombosis. As the diagnosis and management of this syndrome has generated controversy, an updated review of the literature and treatment recommendations may be helpful for providing optimal care for patients with this often unrecognized and confusing infection.

[Lemierre syndrome: several clinical features of "a forgotten disease"]. / Síndrome de Lemierre: distintas presentaciones clínicas de «una enfermedad olvidada¼

INTRODUCTION: Lemierre syndrome (LS) is considered as a forgotten disease since the beginning of Penicillin. The lack of use of beta-lactamase inhibitors antibiotics together with the increase in the use of macrolids or second-generation and third-generation cephalosporins for oropharyngeal processes has led to an increasing incidence. We propose to review all LS cases seen in an Emergency Department over the last 5 years. METHODS: Retrospective study of patients diagnosed as LS in the Emergency Department of Hospital general universitario Gregorio Marañón from 2004 to the present. Descriptive analysis of age, gender, clinical features, laboratory and radiological results, management and outcome. RESULTS: We had 6 patients with LS. 5 males. Median age: 25 years old. All with sore throat and pulmonary embolisms. 2 patients with negative blood cultures. 1 patient was admitted into ICU because of severe sepsis. One of the 6 developed acute renal failure, another one hemoptysis, and another a hydropneumothorax which was drained. All the patients were managed with antibiotics against anaerobes, carbapenems in 3 cases. Two patients did not receive anticoagulants, with no complications recorded. There were no deaths. CONCLUSION: LS still has a high morbidity, therefore it must be on mind in young people with febrile pharyngeal symptoms in order to start specific treatment as soon as possible to decrease complications. Carbapenems appears to be a good therapeutic choice.